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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses

Fig. 4

Abnormal areas in skeletal muscle fibers with protein aggregates in myotilinopathy. Serial skeletal muscle cryosections from a myotilinopathy patient (ID1 in Table 1) were stained with modified Gomori trichrome and double-immunostained with primary antibodies directed against myotilin and filamin C. DAPI was used to visualize nuclei. Three different areas of protein accumulation were detected in abnormal fibers: Abnormal areas A1 appear hyaline on trichrome stain and are characterized by aggregation of myotilin and several other proteins (see Table 3) including filamin C. Abnormal areas A2 are located in the sarcoplasm of abnormal fibers, appear dark blue in trichrome stain and are characterized by a decreased immunoreactivity for myotilin and a strong accumulation of filamin C (among other proteins, see Table 3). Abnormal areas 3 are located around subsarcolemmal vacuoles in abnormal fibers, appear hyaline-purple in trichrome stain and show a decreased immunoreactivity for myotilin and an increased immunoreactivity for filamin C (and other proteins, see Table 3). Scale bar = 20 μm

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